Molecular basis of the t(8;21) translocation in acute myeloid leukaemia

Semin Cancer Biol. 1993 Dec;4(6):369-75.

Abstract

The t(8;21) translocation is one of the most frequent chromosomal abnormalities in acute myeloid leukaemia and results in gene fusion between AML1 on chromosome 21 and MTG8 (= ETO or CDR) on chromosome 8. AML1 contains a region of sequence homology to the Drosophila runt gene and the mouse polyomavirus enhancer binding protein PEBP2 alpha gene. The rearrangement occurs within a specific intron of the AML1 gene and results in the formation of a chimaeric protein with the consistent feature that the region of sequence homology of AML1 is fused with almost the entire MTG8 protein. MTG8 (ETO, CDR) is predicted to be a transcription activation factor from its sequence with zinc-finger motifs and proline-rich domains. Thus the rearrangement is a fusion between two probable transcription activation factors.

Publication types

  • Comparative Study
  • Review

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Chromosomes, Human, Pair 21 / ultrastructure*
  • Chromosomes, Human, Pair 8 / ultrastructure*
  • Consensus Sequence
  • Core Binding Factor Alpha 2 Subunit
  • Core Binding Factor alpha Subunits
  • DNA-Binding Proteins / chemistry
  • DNA-Binding Proteins / genetics*
  • DNA-Binding Proteins / physiology
  • Drosophila Proteins
  • Drosophila melanogaster / genetics
  • Gene Expression Regulation, Leukemic
  • Humans
  • Leukemia, Myeloid, Acute / genetics*
  • Molecular Sequence Data
  • Neoplasm Proteins / chemistry
  • Neoplasm Proteins / genetics*
  • Neoplasm Proteins / physiology
  • Nuclear Proteins
  • Oncogene Proteins, Fusion / chemistry
  • Oncogene Proteins, Fusion / genetics*
  • Oncogene Proteins, Fusion / physiology
  • Protein Binding
  • Protein Structure, Tertiary
  • Proto-Oncogene Proteins*
  • RUNX1 Translocation Partner 1 Protein
  • Sequence Homology, Amino Acid
  • Transcription Factor AP-2
  • Transcription Factors / chemistry
  • Transcription Factors / genetics*
  • Transcription Factors / physiology
  • Transcription, Genetic
  • Translocation, Genetic*

Substances

  • Core Binding Factor Alpha 2 Subunit
  • Core Binding Factor alpha Subunits
  • DNA-Binding Proteins
  • Drosophila Proteins
  • Neoplasm Proteins
  • Nuclear Proteins
  • Oncogene Proteins, Fusion
  • Proto-Oncogene Proteins
  • RUNX1 Translocation Partner 1 Protein
  • RUNX1 protein, human
  • RUNX1T1 protein, human
  • TfAP-2 protein, Drosophila
  • Transcription Factor AP-2
  • Transcription Factors
  • run protein, Drosophila